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Our Team
[Neuropathology associated with dementia].
[sneddon syndrome]
As
dementing
diseases
are
too
numerous
to
refer
to
all
of
them
,
I
confine
my
description
to
the
neuropathology
of
amyotrophic
lateral
sclerosis
with
dementia
(
ALSD
)
,
and
cerebral
vascular
pathology
of
three
unique
vascular
diseases
causing
dementia
.
1
)
ALSD
:
The
cortical
neuropathology
of
this
condition
exhibit
two
main
unique
profiles
in
addition
to
mainly
temporal
lobe-located
cortical
changes
.
One
is
ubiquitin-
positive
intraneuronal
cytoplasmic
inclusions
,
and
the
other
a
localized
neuronal
degeneration
in
the
transitional
zone
between
the
hippocampal
CA
1
and
subiculum
.
2
)
Cerebral
autosomal
recessive
arteriopathy
with
subcortical
infarcts
and
leukoencephalopathy
(
CARASIL
)
:
The
characteristic
vascular
change
of
this
condition
is
marked
intimal
thickening
of
the
middle
and
small
arteries
with
relatively
preserved
smooth
muscle
cells
in
the
media
.
The
scalp
arteries
escape
this
lesion
,
indicating
non-ischemic
pathomechanisms
for
the
baldness
seen
in
this
condition
.
3
)
Cerebral
autosomal
dominant
arteriopathy
with
subcortical
infarcts
and
leukoencephalopathy
(
CADASIL
)
:
The
main
lesions
of
the
cerebral
vessels
are
smooth
muscle
cell
degeneration
of
deep
perforating
and
small
meningeal
arteries
with
deposition
of
granular
osomiophilic
material
in
the
media
of
the
affected
vessels
.
4
)
Sneddon
syndrome
:
This
condition
characterized
by
livedo
reticularis
and
recurrent
multiple
infarctions
shows
marked
sclerotic
changes
in
the
deep
perforating
arterioles
and
main
cerebral
arteries
with
relatively
spared
middle
-
and
small
-sized
meningeal
arteries
.
Diseases
Validation
Diseases presenting
"marked intimal"
symptom
sneddon syndrome
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